Objective:Hydrogen-oxygen nebulization inhalation has a brain-protective effect. This study aims to evaluate its short-term efficacy and safety as an adjuvant treatment for infantile epileptic spasms syndrome (IESS). Methods:A prospective randomized double-blind controlled trial enrolled 53 IESS children (Nov 2021-Nov 2023), randomized via a random number table into a hydrogen-oxygen inhalation group (27 cases, 66.6% H₂/33.3% O₂ nebulization) and an air inhalation group (26 cases, medical air nebulization). Both groups received standard treatment plus 4 daily 1-h interventions for 14 consecutive days. Primary endpoints were spasticity relief and effective rates; secondary endpoints included improvements in high-amplitude abnormal EEG, IL-6 levels, respiratory tract infection rate, and adverse reactions. Statistical analyses used SPSS software. Results:The primary efficacy endpoints (spasm relief rate p = 0.705, effective rate p = 0.950) and secondary efficacy endpoints (disappearance rate of electroencephalogram hypsarrhythmia p = 0.576, change in Kramer score p = 0.140, change in BASED score p = 0.168, abnormal IL-6 rate p = 0.081) of the two groups of children were compared. There was no statistically significant difference. The overall incidence of adverse reactions in the two groups was comparable, and no serious adverse events occurred in either group. However, the incidences of elevated myocardial enzymes (28.3% vs. 10.9%), gastrointestinal dysfunction (43.5% vs. 23.9%), and abnormal liver function (8.7% vs. 0%) in the hydrogen-oxygen inhalation group were significantly higher than those in the air inhalation group (all p < 0.05). Conclusion:Short-term hydrogen-oxygen nebulization inhalation failed to significantly improve the clinical efficacy of standard treatment for children with IESS. Although it is generally safe and feasible, its potential effects on myocardial, liver and gastrointestinal functions of children need to be closely monitored. In the future, large-sample, long-term follow-up multi-center studies need to be carried out to comprehensively evaluate its clinical value. Clinical trial registration:http://www.chictr.org.cn/bin/home, identifier (ChiCTR2100047479).

OBJECTIVE:Evaluate the effect of adding vigabatrin (VGB) following a 7-day assessment of adrenocorticotropic hormone (ACTH) treatment on the initial response in children with IESS. METHODS:After 7 days of ACTH treatment, evaluations were conducted to determine whether VGB should be added to the regimen; children in whom VGB was added formed the sequential treatment group; those continuing ACTH monotherapy served as the control group. We compared initial response rates between the two groups and analyzed the initial response among children who continued to experience epileptic spasms (ES) on the day 7 since ACTH treatment, treated either with the addition of VGB or continued ACTH monotherapy. And recorded the incidence of symptomatic vigabatrin-associated brain abnormalities on MRI (VABAM) in the sequential treatment group. RESULTS:The sequential treatment regimen significantly improved initial response rates compared with monotherapy (78.1 % (25/32) vs. 41.2 % (108/262)). Among children who continued to experience ES on the day 7 since ACTH treatment, sequential VGB combination therapy significantly improved initial response rates compared with ACTH monotherapy (66.7 % (14/21) vs. 13.1 % (23/176)). Multifactorial analysis with propensity score matching supported these findings, showing a higher response rate and greater likelihood of response with sequential treatment compared to that with monotherapy (odds ratio=13.75 (95 % CI, 4.26 - 44.38). Among children receiving sequential ACTH and VGB treatment, 5 developed symptomatic VABAM-like manifestations. CONCLUSION:The combination of ACTH and VGB, while potentially improving initial response rates, may lead to short-term VABAM-like manifestations, which may resolve spontaneously with dose reduction or temporary discontinuation.

OBJECTIVE:This study was undertaken to develop and validate an artificial intelligence (AI) diagnostic tool using hybrid electroencephalographic (EEG)-video signals for automatic epileptic spasms (ES) detection. METHODS:This retrospective cohort study with internal cross-validation and multicenter external validation was conducted from July 2022 to May 2025. It included 252 patients with ES from Chinese PLA General Hospital and 60 from three other medical centers. We developed a multimodal fusion approach combining video and electrophysiological signals. All EEG data were segmented into continuous 4-s pages. The internal cohort consisted of 212 patients (723.4 h video-EEG, 7348 ES, and 643 215 non-ES segments). Clinical validation involved 100 patients across four datasets (212.2 h, 5709 ES and 185 207 non-ES segments) plus 78 controls without ES (218.1 h, 196 249 segments, used for false alarm rate analysis). Primary outcomes included sensitivity, specificity, accuracy, precision, and F1 score versus electroencephalographer interpretation. RESULTS:In internal cross-validation, the hybrid EEG-video model achieved superior performance compared to current EEG-only models (area under the precision-recall curve = .7334, 95% confidence interval [CI] = .7118-.7539, area under the receiver operating characteristic curve = .9820, 95% CI = .9782-.9856). In the clinical validation dataset, the model demonstrated diagnostic sensitivity (.735, 95% CI = .673-.822) and specificity (.995, 95% CI = .992-.996) comparable to experienced electroencephalographers. With AI assistance, all electroencephalographers showed improved sensitivity trends, with one rater showing a marked improvement from .620 (95% CI = .532-.699) to .792 (95% CI = .757-.821). Notably, specificity did not decline significantly for any of the raters. In samples containing subtle ES, machine-assisted recognition significantly improved sensitivity by 16%-21% for all electroencephalographers (p < .01). The model maintained low false alarm rates (.16‰) across different patient populations including healthy controls and epilepsy patients without ES. SIGNIFICANCE:This AI diagnostic tool achieves clinical performance comparable to senior electroencephalographers when applied independently for ES detection, with greater robustness in detecting subtle ES. When used collaboratively with clinicians, it could enhance diagnostic sensitivity while maintaining high specificity. The technology addresses critical diagnostic challenges in pediatric epilepsy care and shows promise to reduce health care inequities in resource-limited settings lacking specialized expertise.

PURPOSE:It is widely believed that electroencephalographers can identify epileptic spasms (ES) accurately. However, additional research is needed to verify this assumption, especially because some ES can be subtle, involving only facial movements, such as eye rolling. METHODS:The EEG data of 22 patients diagnosed with ES (whether or not it is diagnosed as infantile epileptic spasm syndrome) were evaluated by 6 senior electroencephalographers. The content included judgments of the presence or absence of ES throughout the entire examination process for each patient and in segmented pages every 4 seconds and the consistency among electroencephalographers. The inter-rater reliability (IRR) was assessed using the Fleiss kappa statistic. RESULTS:The accuracy of the 6 evaluators for identifying patients with or without ES in the 22-patient data set was 0.727 to 0.90, and the IRR among the 6 raters was moderate (0.45). Moderate IRR was observed among evaluators from tertiary (0.425), and poor IRR was observed among evaluators from nontertiary (0.399) centers. For the 4-second segmented pages, the accuracy for identifying ES in the 22 patients by the 6 evaluators was 0.943 to 1, and the IRR among the 6 evaluators was good agreement (0.63). CONCLUSIONS:Omissions in the identification of ES episodes were noted among different electroencephalographers, and IRR regarding whether a patient experienced an ES or whether a single event constituted an ES was found to be unsatisfactory. Identifying ES remains challenging for even experienced electroencephalographers.

Vocal fold leukoplakia (VFL), a precancerous lesion of the larynx, is characterized by white plaques on the vocal fold mucous membrane. Currently, there are no reliable biomarkers to predict the recurrence and malignant transformation of VFL. Considering chondroitin sulfate proteoglycan 4 (CSPG4) as a biomarker for malignant tumors such as laryngeal squamous cell carcinoma (LSCC), we conducted this cohort study to evaluate the prognostic influence of CSPG4 expression on VFL patients. This study included 44 patients with surgical intervention of VFL. CSPG4 expression in VFL were observed using immunohistochemistry, and the relationship between the prognosis of VFL patients and CSPG4 expression were investigated using Spearman correlation analysis and multiple Cox regression models. During a median follow-up of 89.3 months, recurrence occurred in 19 postsurgical VFL patients, and malignant transformation occurred in 10 patients. The recurrence and malignant transformation group showed significant differences in the H-SCORE of CSPG4 (P = 0.005 and P = 0.045) compared to the the non-recurrence group and the non-malignant transformation group. And the CSPG4 expression level was positive correlation with recurrence and malignant transformation (0.306 or 0.416, spearman correlation confident). Receiver operating characteristic curve analysis indicated that CSPG4 H-SCORE has predictive value for poor prognosis (areas under the curve, 0.742 and 0.710; cut-off points, 82.5 and 177.5, respectively). Multiple Cox regression analysis revealed a statistically significant effect of lesion size (P = 0.014) and CSPG4 H-SCORE (P = 0.005) on recurrence as well as pathological type (P = 0.001) and CSPG4 H-SCORE stratified by 177.5 (P = 0.039). Our survival data indicated that higher CSPG4 expression was associated with a shorter time of recurrence (P = 0.003) and tumorigenesis (P<0.001). CSPG4 overexpression indicates higher risks and shorter time of postoperative VFL recurrence and tumorigenesis. Detection of CSPG4 expression in VFL may be a novel approach to assess the outcome of VFL patients.

OBJECTIVE:Employing whole-exome sequencing (WES) technology to investigate the etiology of infantile epileptic spasm syndrome (IESS), and determining whether different etiologies exhibit phenotypic variations, while elucidating the potential associated factors, might improve short-term responses to first-line treatment. METHODS:We retrospectively evaluated patients with IESS admitted for treatment between January 2018 and June 2023. Clinical phenotypic differences among etiological classifications and clinical manifestations were analyzed. Variable selection using the best subset method was performed, followed by logistic regression analysis to identify the factors influencing treatment response. RESULTS:A total of 577 patients were included; 412 completed trio-WES. Magnetic resonance imaging abnormalities were detected in 387 patients (67.1%). Patients with etiology as structural abnormalities were likelier to have non-spasms at the initial seizure onset. A total of 532 patients completed the first-line treatment; 273 patients received it for the first time at our hospital (initial response rates: 30.1% and 42.1%, respectively). The response group had a lower proportion of early-onset seizures (≤3 months) than the no-response group (11.3% vs. 23.7%, p < 0.01 and 11.3% vs. 21.5%, p = 0.03, respectively). Logistic regression analysis indicated that earlier initiation of first-line treatment was associated with a higher likelihood of an initial response. However, the etiological classification did not have a significant impact on the initial response. INTERPRETATION:IESS patients with structural abnormalities are more likely to present with non-spasm seizures at initial onset. Early initiation of first-line treatment is crucial; however, initial responses may be less favorable when seizures occur in early infancy.

Background This study aimed to investigate the unique electroencephalography (EEG) patterns in neuronopathic Gaucher disease (GD) patients and explore the correlations between EEG findings and neurological phenotypes so as to optimize clinical outcomes.Methods A retrospective analysis was conducted on 74 EEG recordings from 50 GD patients between January 2012 and July 2022.Results Twenty-three patients exhibited abnormal EEG recordings, including 11 of the GD1 type (the transitional type) and 12 with neuronopathic GD. Of the 12 neuronopathic GD patients, 9 patients with epilepsy were analysed specifically in terms of the clinical course. The primary waveform observed in the neuronopathic EEG recordings was the spike-and-wave complex (SWC) during both awake and sleep states. This was significantly different from sharp waves observed only during sleep in the patients of the transitional type (P = 0.0230). The abnormal discharges in the neuronopathic patients were most commonly located in the bilateral Rolandic areas, while the transitional type commonly involved the bilateral frontal regions. Three patients with an epileptic EEG pattern reported their initial seizures years later. Seizures in the neuronopathic patients were effectively controlled with anti-seizure medications (ASMs), despite the ongoing presence of abnormal EEG patterns. The EEG patterns during ocular symptoms were characterized by sporadic or continuous unilateral SWC during sleep.Conclusions Patients with neuronopathic GD exhibit distinct EEG patterns that can help differentiate them from GD1 patients. Early treatment with ASMs can effectively control seizures. EEG plays a crucial role in monitoring seizures and can facilitate prompt intervention for GD patients.

This was a prospective, randomized, double-blind, single-center placebo-controlled trial to assess the efficacy and safety of melatonin as an add-on treatment for infantile epileptic spasms syndrome (IESS). Participants aged 3 months to 2 years with a primary diagnosis of IESS were recruited and assigned to two groups in a 1:1 ratio. Both treatment groups received a combination of adrenocorticotrophic hormone (ACTH) and magnesium sulfate (MgSO4 ) for 2 weeks, and the treatment group also received melatonin (3 mg) between 20:00 and 21:00 daily, 0.5-1 h before bedtime. The study's primary endpoint was the average reduction rate in spasm frequency assessed by seizure diaries. Secondary endpoints included assessment of the response rate, EEG hypsarrhythmia (Kramer score), and psychomotor development (Denver Developmental Screening Test, DDST). Sleep quality was assessed by using the Brief Infant Sleep Questionnaire (BISQ), the Infant Sleep Assessment Scale (ISAS), and actigraphy. Safety parameters were also evaluated. Statistical analyses were conducted on intention-to-treat and per-protocol populations. The trial is registered at Clinicaltrials.gov (ChiCTR2000036208). Out of 119 screened patients, 70 were randomized and 66 completed treatments. In the intention-to-treat population, there were no significant differences in the average percentage reduction of spasm frequency (median [interquartile range, IQR: Q3-Q1], 100% [46.7%] vs. 66.7% [55.3%], p = .288), the 3-day response rate (51.4% vs. 37.1%, p = .229), the 28-day response rate (42.9% vs. 28.6%, p = .212), EEG Kramer scores (2 [3.5] vs. 2 [3], p = .853), or DDST comprehensive months (5 [2.5] vs. 6 [6], p = .239) between the melatonin (n = 35) and placebo (n = 35) groups. However, caregivers reported improved sleep quality after melatonin treatment, with 85.7% reporting regular sleep compared to 42.9% with placebo (42.9%, p < .001). The melatonin group had lower ISAS scores in 4-11-month-old patients compared to the placebo (mean ± SD, 29.3 ± 4.4 vs. 35.2 ± 5.9, p < .001). Moreover, the median (IQR) value of sleep-onset latency was shortened by 6.0 (24.5) min after melatonin treatment, while that in the placebo group was extended by 3.0 (22.0) min (p = .030). The serum melatonin (6:00 h) level (pg/mL) of the children in the melatonin group after treatment was significantly higher than in the placebo group (median [IQR], 84.8 [142] vs. 17.5 [37.6], p < .001). No adverse effects related to melatonin were observed in the study, and there were no significant differences in adverse effects between the melatonin and placebo groups. Although not statistically significant, the results of this randomized clinical trial proved that melatonin supplementation, as an add-on treatment, can improve spasm control rate in the treatment of IESS. For IESS children treated with ACTH, the addition of melatonin was found to improve sleep quality, shorten sleep onset latency, and increase blood melatonin levels. Moreover, it was observed to be a safe treatment option.

Aims The causes of distinct patterns of reduced cortical thickness in the common human epilepsies, detectable on neuroimaging and with important clinical consequences, are unknown. We investigated the underlying mechanisms of cortical thinning using a systems-level analysis. Methods Imaging-based cortical structural maps from a large-scale epilepsy neuroimaging study were overlaid with highly spatially resolved human brain gene expression data from the Allen Human Brain Atlas. Cell-type deconvolution, differential expression analysis and cell-type enrichment analyses were used to identify differences in cell-type distribution. These differences were followed up in post-mortem brain tissue from humans with epilepsy using Iba1 immunolabelling. Furthermore, to investigate a causal effect in cortical thinning, cell-type-specific depletion was used in a murine model of acquired epilepsy. Results We identified elevated fractions of microglia and endothelial cells in regions of reduced cortical thickness. Differentially expressed genes showed enrichment for microglial markers and, in particular, activated microglial states. Analysis of post-mortem brain tissue from humans with epilepsy confirmed excess activated microglia. In the murine model, transient depletion of activated microglia during the early phase of the disease development prevented cortical thinning and neuronal cell loss in the temporal cortex. Although the development of chronic seizures was unaffected, the epileptic mice with early depletion of activated microglia did not develop deficits in a non-spatial memory test seen in epileptic mice not depleted of microglia. Conclusions These convergent data strongly implicate activated microglia in cortical thinning, representing a new dimension for concern and disease modification in the epilepsies, potentially distinct from seizure control.

Even though adrenocorticotropic hormone (ACTH) demonstrated powerful efficacy in the initially successful treatment of infantile spasms (IS), nearly half of patients have experienced a relapse. We sought to investigate whether features of electroencephalogram (EEG) predict relapse in those IS patients without structural brain abnormalities. We retrospectively reviewed data from children with IS who achieved initial response after ACTH treatment, along with EEG recorded within the last two days of treatment. The recurrence of epileptic spasms following treatment was tracked for 12 months. Subjects were categorized as either non-relapse or relapse groups. General clinical and EEG recordings were collected, burden of amplitudes and epileptiform discharges (BASED) score and multiscale entropy (MSE) were carefully explored for cross-group comparisons. Forty-one patients were enrolled in the study, of which 26 (63.4%) experienced a relapse. The BASED score was significantly higher in the relapse group. MSE in the non-relapse group was significantly lower than the relapse group in the γ band but higher in the lower frequency range (δ, θ, α). Sensitivity and specificity were 85.71% and 92.31%, respectively, when combining MSE in the δ/γ frequency of the occipital region, plus BASED score were used to distinguish relapse from non-relapse groups. BASED score and MSE of EEG after ACTH treatment could be used to predict relapse for IS patients without brain structural abnormalities. Patients with BASED score ≥ 3, MSE increased in higher frequency, and decreased in lower frequency had a high risk of relapse.

Infantile spasms are a serious epilepsy syndrome with a poor prognosis. Electroencephalography (EEG) has been a key component in the prognosis and treatment of infantile spasms. This multi-center study protocol is developed to investigate interrater and intrarater agreement of an electroencephalographic grading scale—the Burden of Amplitudes and Epileptiform Discharges (BASED) score among electroencephalographers. Thirty children, aged 0–2 years, with infantile spasms who were hospitalized in the Chinese PLA General Hospital will be recruited into this study by stratified sampling. Seven electroencephalographers from different Class A tertiary hospitals will select a 5-min epoch with the most severe epileptiform discharge, score the EEG reports, and provide the basis for the scoring. The 420 (30 × 7 × 2) scoring results provided by electroencephalographers in two rounds can be analyzed statistically using weighted kappa (weighted $$\kappa$$ ) statstic, Fless’ kappa (Fless’ $$\kappa$$ ) statistic, and intraclass correlation coefficient (ICC) to calculate the interrater and intrarater agreement. We will recruit more electroencephalographers than were included in previous studies to assess the interrater and intrarater agreement in the selection of 5-min EEG epochs, the BASED scores, and the basis for scoring. If the BASED score has an adequate interrater and intrarater agreement, the score will have more significance for guiding the clinical management and for predicting the prognosis of patients with infantile spasms.

Introduction: Even though adrenocorticotropic hormone (ACTH) demonstrated powerful efficacy in the initially successful treatment of infantile spasms (IS), nearly one-half of patients whose spasms were once suppressed experienced relapse. There is currently no validated method for the prediction of the risk of relapse. The Burden of Amplitudes and Epileptiform Discharges (BASED) score is an electroencephalogram (EEG) grading scale for children with infantile spasms. We sought to determine whether an association exists between the BASED score after ACTH treatment and relapse after initial response with ACTH. Methods: Children with IS who achieved initial response after ACTH treatment were selected as the study subjects. Those who experienced relapse within 12 months after ACTH treatment were categorized as the relapse group, and those who did not were categorized as the non-relapse group. Their general clinical data and EEG data (using BASED scoring) after ACTH treatment were collected, and compared between groups. Cox proportional hazards models were fit to determine factors associated with relapse. Results: A total of 64 children with IS were enrolled in the study, of which 37 (57.8%) experienced a relapse, and the median duration after ACTH treatment was 3 (1.5, 6) months. The BASED score was significantly higher in the relapse group than in the non-relapse group. Cox modeling demonstrated that BASED score was independently associated with relapse. The patients with a score greater than or equal to 3 showed a high rate (89.3%) of relapse. The relapse group had stronger, more stable EEG functional networks than the non-relapse group, and there were obvious correlations between BASED score and functional connectivity. Conclusion: This study suggests the BASED score after ACTH treatment has potential value as a predictor for relapse after initial response. Children with IS who have a BASED score greater than or equal to 3 after the initial response of ACTH carry a high risk of relapse within 1 year.

Introduction Infantile spasms (IS) is a type of severe epileptic encephalopathy that occurs in infancy and early childhood. IS is characterised clinically by epileptic spasms, often accompanied by sleep disorder and abnormal circadian rhythm. The endogenous circadian rhythm disorder, in turn, can make spasms worse. Melatonin has also been found to have anticonvulsant and neuroprotective properties by adjusting the circadian rhythm. However, there are lack of relevant studies on controlling IS by using melatonin. This study aims to analyse the therapeutic effect of melatonin supplementation for the treatment of IS. Methods and analysis This is a triple-blinded (trial participant, outcome assessor and the data analyst), prospective, randomised controlled trial to be conducted in the Department of Paediatrics, The First Medical Center of Chinese PLA General Hospital, Beijing, China from November 2020. Patients (n=70) aged 3 months to 2 years with IS will be recruited in this study after receiving written consent from their parents or guardians. Patients will be randomly divided into two equal groups and treated with a combination of adrenocorticotropic hormone, magnesium sulfate and either melatonin or placebo. Clinical data from the patients in the two groups before and after the treatment will be collected and compared. The primary outcome will be assessed 2 weeks later by seizure diaries and reported as the average reduced rate of spasms frequency. Secondary outcomes include the response rate (the rate of spasms-free), electroencephalogram hypsarrhythmia assessment and the psychomotor development assessment (Denver Developmental Screening Test). Sleep quality and safety will also be assessed. Ethics and dissemination The protocol for this study was approved by the Ethics Committee of Chinese PLA General Hospital (reference number S2020-337-01) and was reported according to the Standard Protocol Items: Recommendations for Interventional Trials statement. Findings of this research will be disseminated through national and international meetings, conferences and peer-reviewed journals. Trial registration number ChiCTR2000036208.

Purpose There was no evidence whether the mammalian/mechanistic target of rapamycin pathway hyperactivation and long-term use of mTOR inhibitors have any effects on the physical development of children. The aim was to evaluate these effects by comparing the physical development of children with TSC and normal children. Methods A total of 120 eligible children were enrolled. They were administered sirolimus and followed for at least 12 months. Height, weight, BMI and lipid metabolism index were collected during treatment. Pearson's chi-square and Fisher's exact test were used for comparison of proportions of patients exhibiting normal and abnormal physical growth before and after 1 year of treatment. Logistic regression was used to evaluate the influence of age, sex and abnormal lipid metabolism on the increased BMIs of TSC patients after treatment. Results Most of the enrolled TSC children were in the normal height, weight and BMI ranges at baseline (91.7%, 95.8% and 78.3%, respectively). Most remained in the normal height, weight and BMI ranges after 1 year of sirolimus treatment (94.2%, 95% and 76.7%, respectively). There was no significant difference in the proportion of physical development before and after treatment (p > 0.05). Thirty-eight (38/106, 35.8%) patients had increased BMIs after 1 year of treatment, but there was no significant correlation between age, sex and lipid metabolism and increased BMI. Conclusions Overactivation of the mTOR pathway and long-term administration of sirolimus does not affect the physical development of children with TSC.

BackgroundVagus nerve stimulation (VNS) has been demonstrated to be safe and effective for patients with refractory epilepsy, but there are few reports on the use of VNS for postencephalitic epilepsy (PEE). This retrospective study aimed to evaluate the efficacy of VNS for refractory PEE.MethodsWe retrospectively studied 20 patients with refractory PEE who underwent VNS between August 2017 and October 2019 in Chinese PLA General Hospital and Beijing Children’s Hospital. VNS efficacy was evaluated based on seizure reduction, effective rate (percentage of cases with seizure reduction ≥ 50%), McHugh classification, modified Early Childhood Epilepsy Severity Scale (E-Chess) score, and Grand Total EEG (GTE) score. The follow-up time points were 3, 6, and 12 months after VNS. Pre- and postoperative data were compared and analyzed.ResultsThe median [interquartile range (IQR)] seizure reduction rates at 3, 6, and 12 months after VNS were 23.72% (0, 55%), 46.61% (0, 79.04%), and 67.99% (0, 93.78%), respectively. The effective rates were 30% at 3 months, 45% at 6 months, and 70% at 12 months. E-chess scores before the operation and at 3, 6, and 12 months after the operation were 10 (10, 10.75), 9 (9, 10), 9 (9, 9.75), and 9 (8.25, 9) (P < 0.05), respectively. GTE scores before surgery and at 12 months after the operation were 11 (9, 13) and 9 (7, 11) (P < 0.05), respectively. The mean intensity of VNS current was 1.76 ± 0.39 (range: 1.0–2.5) mA. No intraoperative complications or severe post-operative adverse effects were reported.ConclusionsOur study shows that VNS can reduce the frequency and severity of seizure in patients with refractory PEE. VNS has a good application prospect in patients with refractory PEE.

Infantile spasm is the most common epileptic encephalopathy in infancy.Without early diagnosis and effective treatment, serious consequences and economic burden maybe caused.One of the main characteristics of infantile spasm is the hypsarrhythmia on electroencephalography (EEG). It is important to analyze EEG accurately and reliably by scoring EEG.In order to further improve the EEG scoring system and better play the role of EEG in the diagnosis, treatment and prognosis implications of infantile spasm, in this paper, the advantages and disadvantages of various EEG scoring systems were analyzed, so as to provide clues by summarizing current EEG scoring systems and their applications in clinical practices.

Purpose: This study aimed to analyze the therapeutic effect of sirolimus on seizures in pediatric patients with tuberous sclerosis. Methods: We first compared the efficacy of controlling seizures in all patients after they had taken sirolimus for one year, and then we performed a subgroup analysis based on whether the administered antiepileptic drugs were changed to determine whether the efficacy was associated with changes of antiepileptic drugs. Results: A total of 91 eligible children were enrolled. The response rate was 78.0 % (71/91), and 47.2 % (43/91) of all patients were became seizure-free. The improvement in seizure control before and after treatment with sirolimus was significant (p < 0.001). In the AEDs unaltered group, 34 were responders (34/45, 75.6 %, 95 % CI 17.4-88.3), of which 24 were seizure-free (24/34, 70.6 %). In the AEDs-altered group, 37 were responders (37/46, 80.4 %, 95 % CI 56.7-88.1), of which 19 were seizure-free (19/37, 51.4 %). There was no significant difference between the two groups for reductions in rate of seizure frequency (p = 0.308). In the patients with refractory epilepsy, treatment with sirolimus was also effective (p = 0.01). Logistic regression analysis showed that age was an important factor affecting outcome of epilepsy (p = 0.003, 95 % CI 2.05-38.31). No Grade 3 or 4 adverse events were noted during the follow-up. Conclusions: Sirolimus has a significant effect on seizures associated with tuberous sclerosis complex (TSC), with no or only moderate adverse events after long-term administration. Sirolimus could be used as the first-line medication for pediatric patients with TSC-associated epilepsy.